Teprotumumab-associated persistent unilateral hearing loss in dysthyroid optic neuropathy: a case report with review of the literature
Abstract
We report a case of severe thyroid eye disease (TED) complicated by dysthyroid optic neuropathy (DON) in a 55-year-old woman with Graves' disease. Despite insufficient response to high-dose intravenous steroid therapy, teprotumumab led to marked improvement in proptosis, orbital inflammation, and visual function. However, treatment was associated with progressive and persistent sensorineural hearing loss after completing eight infusions. Auditory symptoms began after the fifth infusion with intermittent aural fullness and sound reverberation, progressing to mild down-sloping sensorineural hearing loss. Nine days after the final infusion, the four-frequency pure-tone average (PTA4) was 21.25 dB hearing level (HL) in the right ear and 27.50 dB HL in the left ear. Despite initiation of oral prednisolone therapy (40 mg/day with tapering), progressive hearing loss was observed in the right ear, particularly in the low- to mid-frequency range (250-2,000 Hz), with thresholds worsening to 90 dB HL. At twenty days after the final infusion, PTA4 had increased to 75.00 dB HL in the right ear and 31.25 dB HL in the left ear. Serial audiometric assessments at 107, 128, and 198 days after the final infusion demonstrated no recovery. The patient's history of sudden sensorineural hearing loss suggests increased susceptibility to teprotumumab-associated ototoxicity. This case underscores the importance of individualized risk assessment, careful audiologic monitoring, and shared decision-making when balancing visual recovery against the risk of potentially irreversible auditory toxicity.
Related
- Teprotumumab for dysthyroid optic neuropathy: early functional and structural improvement in a retrospective case series
- Characteristics and Management of a Cohort of Patients with Asymmetric Thyroid Eye Disease and Unilateral Dysthyroid Optic Neuropathy Undergoing Unilateral Orbital Decompression Surgery
- Thyroid eye disease with dysthyroid optic neuropathy 15 years after incomplete thyroidectomy
- Unilateral nonarteritic anterior ischemic optic neuropathy associated with obstructive sleep apnea-hypopnea syndrome: a case report.
- The utility of computed tomography and magnetic resonance imaging in the diagnosis and surgical planning of thyroid eye disease and dysthyroid optic neuropathy
BibTeX
@article{hiromatsu2026teprotumumabassociated,
title = {{Teprotumumab-associated persistent unilateral hearing loss in dysthyroid optic neuropathy: a case report with review of the literature}},
author = {Yuji Hiromatsu and Kumiko Suzuki and Shiho Watanabe and Takahiro Fukuyama and Narihito Tatsumoto and Satoe Nakamura and Yoko Sagara and Yuko Akehi and Eiji Kawasaki and Hiroki Kojima and Kimihiko Sakai},
journal = {Endocrine Journal},
year = {2026},
month = nov,
doi = {10.1507/endocrj.ej26-0064},
url = {https://www.jstage.jst.go.jp/article/endocrj/73/11/73_EJ26-0064/_article},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Teprotumumab-associated persistent unilateral hearing loss in dysthyroid optic neuropathy: a case report with review of the literature
AU - Yuji Hiromatsu
AU - Kumiko Suzuki
AU - Shiho Watanabe
AU - Takahiro Fukuyama
AU - Narihito Tatsumoto
AU - Satoe Nakamura
AU - Yoko Sagara
AU - Yuko Akehi
AU - Eiji Kawasaki
AU - Hiroki Kojima
AU - Kimihiko Sakai
PY - 2026
DA - 2026/11/02
JO - Endocrine Journal
DO - 10.1507/endocrj.ej26-0064
UR - https://www.jstage.jst.go.jp/article/endocrj/73/11/73_EJ26-0064/_article
AB - We report a case of severe thyroid eye disease (TED) complicated by dysthyroid optic neuropathy (DON) in a 55-year-old woman with Graves' disease. Despite insufficient response to high-dose intravenous steroid therapy, teprotumumab led to marked improvement in proptosis, orbital inflammation, and visual function. However, treatment was associated with progressive and persistent sensorineural hearing loss after completing eight infusions. Auditory symptoms began after the fifth infusion with intermittent aural fullness and sound reverberation, progressing to mild down-sloping sensorineural hearing loss. Nine days after the final infusion, the four-frequency pure-tone average (PTA4) was 21.25 dB hearing level (HL) in the right ear and 27.50 dB HL in the left ear. Despite initiation of oral prednisolone therapy (40 mg/day with tapering), progressive hearing loss was observed in the right ear, particularly in the low- to mid-frequency range (250-2,000 Hz), with thresholds worsening to 90 dB HL. At twenty days after the final infusion, PTA4 had increased to 75.00 dB HL in the right ear and 31.25 dB HL in the left ear. Serial audiometric assessments at 107, 128, and 198 days after the final infusion demonstrated no recovery. The patient's history of sudden sensorineural hearing loss suggests increased susceptibility to teprotumumab-associated ototoxicity. This case underscores the importance of individualized risk assessment, careful audiologic monitoring, and shared decision-making when balancing visual recovery against the risk of potentially irreversible auditory toxicity.
SN - 1348-4540
SN - 0918-8959
ER -